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Long-term results of operation for IHSS were reviewed in 124 patients operated upon between 1960 and 1975. Most patients improved symptomatically and manifested marked reduction in LV outflow gradient at rest postoperatively. However, ten (8%) patients died of causes related to operation, 14 (12%) had persistent or recurrent severe functional limitation, and 11 (9%) died up to 13 years postoperatively due to hypertrophic cardiomyopathy. Overall annual mortality rate was 3.5% and was 1.8% for late deaths alone. Of I I late postoperative deaths, six were sudden and five were due to chronic heart failure. Atrial fibrillation was significantly more common in patients who died late postoperatively than in survivors. Nine of the 11 late deaths had associated medical problems that may have contributed to their outcome. In conclusion, long-lasting clinical improvement occurred in most patients who survived operation for IHSS. However, 12% of patients deteriorated clinically over the 5.2 year average follow-up, and there is continued, small annual mortality.